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Published on: July 1, 2012
Living-related liver transplantation for Crigler-Najjar syndrome in Saudi Arabia
Haider Al Shurafa1, Sami Wali, May S Chehab
1Department of Surgery, Riyadh Armed Forces Hospital, Riyadh, Kingdom of Saudi Arabia.
Insights
Living-related liver transplantation offers a cure for Crigler-Najjar syndrome type I, a genetic liver disorder. Early LRLT in Saudi Arabia prevented neurological damage and eliminated the need for phototherapy in transplanted children.
Area of Science:
- Hepatology
- Pediatric Gastroenterology
- Transplant Surgery
Background:
- Crigler-Najjar syndrome type I (CNS-I) is a rare genetic disorder causing severe unconjugated hyperbilirubinemia.
- The syndrome often leads to neurological damage (kernicterus) and is fatal without treatment.
- Phototherapy is a temporary measure, and liver transplantation is the only curative option.
Purpose of the Study:
- To analyze the outcomes of six children with CNS-I.
- To report the first living-related liver transplants (LRLT) for CNS-I in Saudi Arabia and the Middle East.
- To evaluate LRLT as a treatment for CNS-I in a region with limited cadaveric organ availability.
Main Methods:
- Retrospective review of medical records for six children diagnosed with CNS-I.
- Detailed analysis of three cases undergoing LRLT between November 1998 and January 2001.
- Monitoring of pre- and post-transplant bilirubin levels, complications, and need for phototherapy.
Main Results:
- Three children with severe hyperbilirubinemia (362-502 micromol/L) underwent successful LRLT, eliminating the need for phototherapy.
- Post-transplant complications included acute hepatocellular rejection (treated with methylprednisolone), cytomegalovirus infection (treated with ganciclovir), and a biliary leak (surgically repaired).
- Untransplanted patients or those awaiting transplant developed neurological damage or remained on phototherapy, highlighting the urgency of early intervention.
Conclusions:
- Living-related liver transplantation is a curative and optimal treatment for Crigler-Najjar syndrome type I, especially in regions with organ shortages.
- Early LRLT, before neurological deficits manifest, is crucial for preventing kernicterus and achieving favorable outcomes.
- CNS-I appears relatively common in Saudi Arabia, underscoring the importance of LRLT programs for this condition.
Objective:
To analyse the outcome of six children with Crigler-Najjar syndrome type I (CNS-I) and report the first three living-related liver transplants for this syndrome in Saudi Arabia and the Middle East.
Settings:
To review the medical records of six children suffering from CNS-I, three of whom underwent living-related liver transplantation (LRLT) between 22 November 1998 and January 2001.
Main Results:
Living-related liver transplantation was performed in three children with a pre-transplant unconjugated bilirubin level of 362, 381 and 502 micromol/L, respectively, despite daily phototherapy of >or= 12 h. Two of the transplanted children developed acute hepatocellular rejection, which was successfully treated with methylprednisolone pulse therapy. One tested cytomegalovirus positive (using the PP65 method), but showed no signs of clinical infection and was treated with ganciclovir. One patient had a biliary leak at the cut surface of the graft which was surgically repaired. Post-operative bilirubin levels returned to normal in all three transplanted children and no further phototherapy was required. One patient, who was not transplanted but received phototherapy, developed severe neurological damage prior to the start of our living-related liver transplant programme with a bilirubin level of 450 micromol/L, her sister is still awaiting transplantation. A 14-yr-old child with a bilirubin level of 420 micromol/L is presently undergoing phototherapy whilst awaiting orthotopic liver transplantation because of the lack of a suitable living-related donor. Six siblings of the six children in our series were reported dead by the families.
Conclusion:
Crigler-Najjar syndrome type I is a relatively common disease in Saudi Arabia for which LRLT is a curative treatment when performed at an early age before the development of kernicterus and neurological deficiency. In countries where there is a severe shortage of cadaveric organs, as is the case in Saudi Arabia, LRLT is the optimum treatment modality for this syndrome.
